Sybile Val, MD Department of Surgery. October 29, 2009

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1 Soft Tissue Tumors Sybile Val, MD Department of Surgery SUNY Downstate Medical Center October 29, 2009

2 Case Presentation 52 YOM presented to ED on 7/22 with 52 YOM presented to ED on 7/22 with abdominal pain

3 Case Presentation PMH: Schizophrenia PSH: None Meds: Risperdal ALL: NKDA SocHx: ½ PPD x 20 yrs Jehovah s Witness

4 Case Presentation Vital: / % Exam: Diffusely tender Labs:

5 Hospital Course HD #1 GI consult Surgery consult HD#2 EGD Colonoscopy VIR biopsy HD#3 Started on diet HD#4 Tachycardic HD#5 Transferred to surgical service Spiked to Cultures and CBC Hct: 17 Path: Malignant small round blue cell tumor with extensive necrosis and degenerative changes

6 Hospital Course HD#6 Started on Epogen HD#10 Discharged home with surgical follow up Surgical lclinici Pt asymptomatic Hct increased to 30 Scheduled for resection

7 Hospital Course 8/23/09 Pt admitted for bowel prep 8/24/09 Exploratory laporatomy Evacuation of hemoperitoneum Resection of small bowel mesenteric mass Small bowel resection x 2 Partial omentectomy Cholecystectomy y

8 Intra-Operatively

9 Intra-Operatively

10 Intra-Operatively

11 Post-Operatively POD#0 Hypotensive in RR Central line placed Started on pressors POD#1 Weaned offallpressors Extubated in afternoon POD#3 NGT removed Hct 19 POD#4 Started on clears Iron and Epogen resumed POD#10 Hct 23 POD#11 Discharged home

12 Pathology Abdominal Mass 40x20x8cm in aggregate (19x11x5) High gradespindle cell sarcoma

13 While VA POD #49 Presented to ER with 3 days of abdominal pain associated with nausea and vomiting AVSS Exam: LLQ pain Labs:

14

15

16 Hospital Course Admitted to surgical service Conservative management x 2 days Taken to the OR 8/15/09 Exploratory laporatomy carcinomatosis encountered Evacuation of hemoperitoneum (500ml) Lysis of adhesions

17 Hospital Course POD#6 Extubated POD#7 Started on clears Made DNR/DNI by family Currently Tolerating regular diet Awaiting hospice

18 Questions??

19 Soft Tissue Tumors

20 Soft Tissue Sarcoma Collective term for diverse group of malignancies Arise from mesoderm Represent 1% of all adult tumors Represent 15% of pediatric tumors Occur anywhere in the body 43% extremities 15% retroperitoneum 13% viscera 10% trunk More than 50 distinct histological subtypes

21 Soft Tissue Sarcoma Etiology toogyis unclear cea Tumor grade is the best indicator of Biological aggressiveness Metastatic potential Grade is defined by: Tumor cellularity Nuclear atypia Degree of necrosis Mitotic activity Present as asymptpmatic mass

22 Indications for biopsy: Mass larger than 5cm Historyof of growth Persists for more than 4 weeks SURGERY is the FOUNDATION of the treatment of STS

23 For intra-abdominal or retroperitoneal sarcomas: FNA or CNB is not indicated Except: Suspicion for lymphoma or germ cell tumor Tissue diagnosis for preoperative therapy Tissue dx for unresectable disease Suspected metastasis t from another primary

24 Poor prognostic factors: Large size Deep seated Retroperitoneal vs. extremity High grade High stage Positive i margins

25

26 Specific Therapy by Site Extremity (43%) Surgery alone Amputation Limb Sparing Surgery and adjuvant radiation Standard of care Retroperitoneal and intra abdominal (15%) Surgery with negative margins Chemotherapy Radiation (??) Adjuvant Preoperative Intra abdominal

27 Regional Lymphadenectomy Not indicated Only 26%of sarcomas metastasize to lymph Only 2.6% of sarcomas metastasize to lymph nodes

28 Treatment Options Radiation Therapy Recommended for high grade resected STS Improves local recurrence No impact on survival Optimal timing unclear Chemotherapy Controversial Favorable px factors for response to chemo: Young Age Good performance status Absence of liver mets Liposarcoma or synovial histology

29 Recurrence Rates: Extremity 8 20% Retroperitoneum 38 50%

30 Could we have done something different in this case??

31 Retrospective Analysis Included only patients with advanced disease Primary tumor or local recurrence not amendable to complete surgical resection Presence of metastatic disease Goal: Determine the efficacy of palliative chemotherapy Responders: those who achieved a complete or partial response Non responders: All others Overall survival: measured from start of chemo until death/last follow up Determine whether specific factors influence the outcome of chemotherapy

32

33 Median time to progression was 3 months

34 23% of pts were 2 years

35 189/488 (39%) 299/488 (61%)

36 Concluded: Palliative chemotherapy in advanced STS should be regarded as a standard treatment option in the management of these aggressive tumors Doxorubicin based combinations regimens may be associated with superior survival to that achieved with single agent Factors predictive of superior survival included Younger age Absence of bone mets Synovial or liposarcoma histology

37 Surgical resection is the most effective potentially curative therapy for soft tissue sarcomas regardless of origin

38 In Summary Soft tissue sarcomas arise from mesenchymal tissues Extremities and retroperitoneum are most common sites MRI is preferred ddiagnostic i imaging i modality Biospy may be considered for extremity lesions Surgical resection is mainstay of treatment

39 Thank You

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